Universidade Federal de Minas Gerais (UFMG), Programa de Pós-Graduação em Saúde da Criança e do Adolescente, Belo Horizonte, MG, Brazil.
Universidade Federal de Minas Gerais (UFMG), Faculdade de Medicina, Departamento de Pediatria, Belo Horizonte, MG, Brazil.
J Pediatr (Rio J). 2019 Jul-Aug;95(4):451-457. doi: 10.1016/j.jped.2018.04.012. Epub 2018 Jun 27.
To evaluate the cognitive abilities of children and adolescents with sickle cell anemia diagnosed through neonatal screening and to compare them with healthy controls, adjusting the results to their socioeconomic status.
Cognitive assessment was performed with the Wechsler WISC-III scale in 64 children and adolescents with sickle cell anemia and in 64 controls matched by gender and age, without the disease and without neurological impairment; socioeconomic status was measured by the Criterion Brasil.
All cognitive scores were lower in the group of patients. The mean overall IQ, Verbal IQ, and Performance IQ were, respectively, 90.95 for the group of patients and 113.97 for the controls (p<0.001); 91.41 for the group of patients and 112.31 for the controls (p<0.001); 92.34 for the group of patients and 113.38 for the controls (p<0.001). Scores for processing speed, distraction resistance, and perceptual organization were also significantly lower in patients. A direct and significant correlation was detected between socioeconomic status and cognitive scores. In the multivariate analysis, for the same socioeconomic status, a child with sickle cell anemia had an average IQ of 21.2 points lower than the mean IQ observed for the controls (p<0.001), indicating that the disease, adjusted for the socioeconomic effect, is a strong predictor of the overall IQ.
The cognitive impairment of children with sickle cell anemia is severe and manifests even when the disease effect is adjusted to the socioeconomic status. In the authors' view, such impairment requires an early preventive approach in order to avoid this cognitive damage.
评估通过新生儿筛查诊断为镰状细胞贫血的儿童和青少年的认知能力，并与健康对照组进行比较，同时调整结果以适应其社会经济状况。
使用韦氏 WISC-III 量表对 64 名镰状细胞贫血儿童和青少年以及 64 名性别和年龄匹配、无疾病和无神经损伤的对照组进行认知评估；社会经济状况采用 Criterion Brasil 进行评估。
所有认知评分均低于患者组。患者组的平均总智商、言语智商和操作智商分别为 90.95，对照组为 113.97（p<0.001）；患者组为 91.41，对照组为 112.31（p<0.001）；患者组为 92.34，对照组为 113.38（p<0.001）。患者组的处理速度、抗分心和知觉组织评分也明显较低。社会经济地位与认知评分之间存在直接且显著的相关性。在多变量分析中，对于相同的社会经济地位，患有镰状细胞贫血的儿童的平均智商比对照组观察到的平均智商低 21.2 分（p<0.001），这表明该疾病在调整社会经济影响后是整体智商的一个强有力预测因素。
镰状细胞贫血儿童的认知障碍严重，即使在调整疾病对社会经济状况的影响后也会出现。作者认为，这种认知障碍需要早期采取预防措施，以避免这种认知损害。
